2014
Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS
Hadzipasic M, Tahvildari B, Nagy M, Bian M, Horwich AL, McCormick DA. Selective degeneration of a physiological subtype of spinal motor neuron in mice with SOD1-linked ALS. Proceedings Of The National Academy Of Sciences Of The United States Of America 2014, 111: 16883-16888. PMID: 25385594, PMCID: PMC4250117, DOI: 10.1073/pnas.1419497111.Peer-Reviewed Original ResearchMeSH KeywordsAmyotrophic Lateral SclerosisAnimalsMiceMotor NeuronsPatch-Clamp TechniquesSpinal CordSuperoxide DismutaseSuperoxide Dismutase-1ConceptsMN cell bodiesMotor neuronsMo of ageCell bodiesSpinal cordSpinal cord tissue slicesWhole-cell patch-clamp recordingsCell patch-clamp recordingsAdult mouse spinal cordIntrinsic electrophysiologic propertiesTwitch muscleSpinal motor neuronsLower extremity musclesTransgenic mouse modelMouse motor neuronsPatch-clamp recordingsAmyotrophic lateral sclerosisMouse spinal cordSlow-twitch muscleSteady-state firing ratePhysiological subtypesRetrograde tracingAcute slicesExtremity musclesPathophysiologic events
2013
Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm
Song Y, Nagy M, Ni W, Tyagi NK, Fenton WA, López-Giráldez F, Overton JD, Horwich AL, Brady ST. Molecular chaperone Hsp110 rescues a vesicle transport defect produced by an ALS-associated mutant SOD1 protein in squid axoplasm. Proceedings Of The National Academy Of Sciences Of The United States Of America 2013, 110: 5428-5433. PMID: 23509252, PMCID: PMC3619309, DOI: 10.1073/pnas.1303279110.Peer-Reviewed Original ResearchMeSH KeywordsAnimalsAxonal TransportBacterial ProteinsDecapodiformesGene Expression ProfilingHSP110 Heat-Shock ProteinsHumansLuminescent ProteinsMAP Kinase Kinase Kinase 5MiceMice, TransgenicMutation, MissenseP38 Mitogen-Activated Protein KinasesPhosphorylationProtein FoldingProteomicsRecombinant Fusion ProteinsSpinal CordSuperoxide DismutaseSuperoxide Dismutase-1Transport VesiclesConceptsMutant SOD1 proteinTransport defectSOD1 proteinApoptosis signal-regulating kinase 1 inhibitorsPhosphorylation of kinesinNucleotide exchange factorsMolecular chaperone Hsc70Different oligomeric statesYellow fluorescent proteinTransgenic miceProtein disaggregationChaperone Hsc70Exchange factorMAPK cascadePhosphorylation of p38Oligomeric stateDiminished phosphorylationKinase 1 inhibitorFusion proteinFluorescent proteinP38 inhibitorP38 MAPKPhosphorylationHsp110Cu/ZnRNA-Seq Profiling of Spinal Cord Motor Neurons from a Presymptomatic SOD1 ALS Mouse
Bandyopadhyay U, Cotney J, Nagy M, Oh S, Leng J, Mahajan M, Mane S, Fenton WA, Noonan JP, Horwich AL. RNA-Seq Profiling of Spinal Cord Motor Neurons from a Presymptomatic SOD1 ALS Mouse. PLOS ONE 2013, 8: e53575. PMID: 23301088, PMCID: PMC3536741, DOI: 10.1371/journal.pone.0053575.Peer-Reviewed Original ResearchConceptsRNA metabolismMutant proteinsMotor neuron cell bodiesPost-translational effectsRNA-seq profilingMotor neuronsNeuron cell bodiesWhole transcriptome profilingMonths of ageAltered expression levelsMutant human SOD1Transcriptome profilingLower motor neuron cell bodiesCell bodiesTransgenic strainsSpinal cord motor neuronsSymptoms of ALSMutant SOD1 miceSOD1-ALS miceAge-matched animalsHuman SOD1Amyotrophic lateral sclerosisProtein TDP43Expression levelsForms of ALS